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From orthostatic hypotension to Shy-Drager syndrome
  1. J M S Pearce
  1. 304 Beverley Road, Anlaby, East Yorkshire HU10 7BG, UK; jmspearcefreenet.co.uk

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    Syncope derives from the Greek synkoptein, meaning to strike, cut off, or weary. Hippocrates and biblical texts describe victims of fainting. In the USA syncope accounts for 3% of emergency room visits and 1–6% of all hospital admissions,1 Pierre Adolph Piorry2 (1794–1879) in 1826 reported,

    “When a patient faints, symptoms improve when he is laid flat.”3

    Piorry was a pioneer of percussion and pleximetry, best remembered for his work in chest diseases and for coining the term “uraemia”.

    Thomas Addison, when describing postural syncope in adrenal failure, also noted:

    “Attacks of giddiness and dimness of sight…would occur always when in the standing posture and were instantly relieved by sitting or lying down.”

    Bradbury and Eggleston4 first recorded a recurrent fainting of unknown cause in 1925 in three patients subject to attacks of idiopathic orthostatic hypotension. Many lone and familial cases have subsequently appeared in the literature.5

    Orthostatic hypotension in association with other neurological disease occurs in tabes, syringomyelia, spinal cord injury, and in polyneuropathies, Parkinsonism, multiple system atrophy (MSA), and a variety of cerebrovascular and tumourous lesions. In 1960 Milton Shy at the NIH and Glen Albert Drager of Houston described comprehensively the clinicopathology of MSA:

    “A neurological syndrome associated with orthostatic hypotension…which is of adult onset and consists of orthostatic hypotension, bladder and bowel incontinence, anhidrosis, iris atrophy, amyotrophy, ataxia, rigidity and tremor; intellect is unaffected”6

    Schatz later revised7 the nosology for autonomic disorders: (1) primary, autonomic failure (idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome) with no neurologic defects other than autonomic dysfunction; (2) multiple system atrophy, equivalent to Shy-Drager syndrome (a sporadic, progressive, adult onset disorder characterised by autonomic dysfunction, parkinsonism, and ataxia in any combination); and (3) secondary autonomic failure delineates diseases in which the cause is known (for example diabetes, amyloidosis, dopamine beta-hydroxylase deficiency, and drug side effects).

    The American Autonomic Society has defined multiple system atrophy as:

    “A sporadic, progressive, adult onset disorder characterized by autonomic dysfunction, Parkinsonism, and ataxia (a failure of muscular coordination) in any combination.…The main features are: Parkinsonism, Cerebellar signs, Autonomic Failure, Striatonigral Degeneration, Sporadic Olivo-ponto-cerebellar Atrophy, Shy-Drager Syndrome.”

    George Milton Shy (1919–1967) qualified in Oregon in 1943 and after brief war service, in which he was wounded, studied neurology at the Montreal Neurological Institute, and at the National Hospital, Queen Square. Unusually for an American, he became MRCP London by examination, in 1947. He carried out highly regarded research on myopathies and published extensively. His work on the Kearns-Sayre syndrome, oculopharyngeal myopathy (Shy-Gonatas syndrome), and a myopathy of infants (Shy-Magee syndrome) are lasting monuments to his protean interests. In 1953 he became clinical director of the National Institute of Neurological Diseases and Blindness, Bethesda, and in 1962 chairman of neurology in Pennsylvania, before taking the chair and directorship of the New York Neurological Institute, Columbia in 1967. Aged only 47 he died suddenly a few weeks later.

    Little is recorded about Drager (1917–1967), a talented physician at Baylor College of Medicine, Houston, who also died young, in the same year as Shy.

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